A Woman's Journey With Rare Disease

Deeper Questions

I had shared this news article to Facebook awhile ago for a few reasons. For one, I was excited that my center (Hospital of the University of Pennsylvania) already has & utilizes this technology. They are a hospital that is ranked 10th in Pulmonology in the Nation (source: US News & World Report – Best Hospitals) but also they have one of the oldest transplant centers with highest outcomes post transplant. Given the risk of double lung transplants, this makes me feel more secure. Even the centers in NY do not have the track record that Upenn has. This is amazing to me because one of my main fears when my friends & I were moving to the Lehigh Valley in 2010 was where I could continue my medical care since both TSC & LAM are rare diseases & I wondered if I would I be in as good of hands as I was in NY. I feel the honest answer is I’m in better.

Yet there was an important question I’d soon have to consider for myself outside of this article. How would I feel about being transplanted with “marginal” lungs?

Being a realist, I know I can’t afford to be picky if I get the opportunity. Marginal in this case doesn’t mean bad. It means that the death of the donor was due to a cause like suffocation or drowning which puts them at a higher risk or at least perceived higher risk of complication than the average donor lung. But with this technology, not only can the lungs be made more viable for a longer period of time, they can be monitored better & any damage actually reversed. In looking deeper, I have found several studies that report the risks from marginal lungs are no greater or less than “regular” donor lungs. I see this as a way too to make more organs viable to people who really need them. The honest answer, if they were recommended to me, & I was advised beforehand I would take them.

However, I can see people’s hesitancy both in connotation & belief. Everyone is hoping for the “perfect set” of lungs for them, & rightfully so. Everyone is also afraid of the unknown. But the connotation for some people over the word marginal is too much to overcome. I will point out that many transplant patients who are awaiting kidneys because they don’t have a family donor match are also facing these same questions.

But the fact remains – on whatever side of the fence one lands on – we need more viable organs. Prior to this technology being developed how many organs & how many lives were lost because there wasn’t a way to make “marginal” lungs viable or assess their performance? How many who could have been given a window & second chance, missed it because they weren’t even given the choice?

Take a look at this article & you’ll see what people who are waiting are up against in conquering the wait time. The quotes come from the physician are from my transplant pulmonologist at UPenn, a man I have come to highly respect both in skill & compassion, Dr. Ahya.

The cold hard truth is too, that not everyone who gets evaluated will qualify. Not everyone who qualifies will get one. Not everyone who gets one, survives or doesn’t have complications post-transplant. But yet, it is still worth the risk. There are many that do extremely well.

While not on your deathbed imminently when listed (unless you fall suddenly ill), not only does one have to live with the knowledge that in order to get an organ, someone has to die, which is something to wrestle with in & of itself, but then a lot depends on the scoring & where you fall on the list as to when it will happen. (Because the wait times are longer for certain organs they tend to list sooner, then push you up the list the sicker you get).

There is a balance too because you can’t become so sick you can’t make it through the evaluation testing or through the surgery. I knew people who were so afraid to have the conversations or to get tested that they missed the window for either. Luckily for me, my local pulmonologist for a long time (who is now sadly moving to Florida but has left me with a great colleague to take over) said this was a major concern of his based on past patients he had & urged me to go early for the initial facts & figures and “real talk”. I hesitated at first because I wasn’t sure what the point was if I was way too healthy, but now I’m really glad I did. This would be a greater elephant in the room to deal with & to approach from first talk-to testing- to listing potentially all in one-shot (or two), than build a relationship over time & take things as they come (which is what I have been able to do). So I can feel confident in the timing & decisions of all of this in my case.

I have a lot of factors, that make other centers hesitant to take me on, others may still feel my scores would be too high to list. Maybe even UPenn will determine that after my testing in August. It’s a lot to sit with & with a lot of unknowns that have driven me crazy at points if I sit there & ponder it too much. Because it’s more than just numbers, or a score – there’s a lot of quality-of-life decisions that factor in.

I have a few advantages in that women with LAM tend to fair way better than national averages post transplant. I have a few disadvantages because I still do have some mild kidney disease from TSC which increases the risk slightly as well as the fact that I had to have my lungs glued to my chest wall to keep them from collapsing which poses a longer & more difficult surgery. But that kidney issue was one of the major things that I discussed with Dr. Ahya in 2010 & also what I figured might knock me out of contention in the first place. Because a stress like transplant surgery can cause failure of other organs so they have to be honest in assessing all potential risks & complications. But the Rapamune has kept that in check & kept it mild so it’s less of a concern than past years to my relief. My tumors have actually shrunk from what they were prior to me using the drug, so I keep my fingers crossed that that will remain the case & am grateful for that. However, if they still feel I am too healthy to list, then I’m going to have to come up with new strategies to manage the lung decline because even 2 points at this level is more significant than 10 point loss at my old level. I’ve made a lot of adaptations but this is testing my creativity & resources at points.

I do take comfort in the fact that I have bipartisan support from my Senate delegation in Congress for pushing for reforms that should make donation easier & potentially increase the donor pool. Both Bob Casey & Pat Toomey have supported legislation that have given people a chance they might not have gotten otherwise. What I didn’t realize, was that for Casey, the issue is personal.

For people who don’t like that I’m getting a tad political here, I point this out because there are a lot of ethical & personal stories that drive home the obstacles, political discussions, & controversies that arise over scoring & who actually gets organs & when. There are also many misconceptions over the process itself that need to be addressed & debunked to make sure donors feel confident with their choices when they opt-in or that people who are on the fence, don’t opt-out over false truths.

I know I am only starting to scratch the surface on so much of it. But then, I also knew that this whole overarching subject was one worth blogging about; showing the human & personal side of it. Not any easy thing, but an essential thing. So when anyone else is faced with this decision maybe they can make an educated choice for themselves in concert with their family, friends, & care team but in order to do that they’ll have to think about many things.